Levels of the NFH and NFL have been reported to be significantly elevated in the CSF of ALS than in healthy controls and patients without parenchymal CNS disease, indicating axonal impairment in ALS [8, 53, 54] With regards to blood, NFL has appeared in serum-derived EVs with a validity of 84–100% and specificity of 76–97%, whereas the data for NFH in serum has been 61–80% for validity and 72.1–83.7% for specificity, implying that serum NFH seems to be slightly a less potent biomarker [55]. This evidence concerns the gene NEFH and amyotrophic lateral sclerosis.